Made as a part of residency program in Dermatology. concise information about mucinosis.

1. CUTANEOUS
MUCINOSIS
D R . S A B H A TA L I B N E A Z E E

2. MUCIN
• Protein or hyaluronic acid complex, produced by fibroblasts
• Capable of absorbing 1000 times its own weight in water.
• Increased mucin= oedema

3. CLASSIFICATION
A. Primary
I. Dermal mucinoses
I. Lichen myxoedematosus (papular mucinosis)
I. Generalized and sclerodermoid lichen myxoedematosus
II. Localized lichen myxoedematosus
a) Acral persistent papular mucinosis
b) Discrete papular lichen myxoedematosus
c) Cutaneous (papular) mucinosis of infancy
d) Nodular lichen myxoedematosus

4. II. Reticular erythematous mucinosis
III. • Scleredema
IV. • Myxoedema in thyroid disease
a) Localized (pretibial) myxoedema
b) Generalized myxoedema
V. • Papular and nodular mucinosis in connective tissue diseases
VI. • Self‐healing cutaneous mucinosis
VII. • Cutaneous focal mucinosis
VIII.• Digital myxoid (mucous) cyst

5. II. Follicular mucinoses
1. Pinkus follicular mucinosis (alopecia mucinosa)
2. Urticaria‐like follicular mucinosis
B. Secondary
1. Epidermal
a) Mycosis fungoides
b) Keratoacanthoma
c) Basal cell carcinoma

6. 2. Dermal
a) Granuloma annulare
b) Connective tissue diseases (lupus erythematosus, dermatomyositis
c) Degos disease
d) Nephrogenic systemic fi brosis
e) Hereditary progressive mucinous histiocytosis
f) Obesity‐associated lymphoedematous mucinosis
g) Hypertrophic scars

7. h) Chronic graft‐versus‐host disease
i) Cutaneous reactions to interferon
j) Herpes zoster
k) Stasis dermatitis due to venous insufficiency
l) Epithelial tumours (basal cell carcinoma)
m) Mesenchymal tumours (dermatofi brosarcoma protuberans)
n) Neural tumours (neurofibroma)

8. 3. Follicular
a) Haematological malignancies (mycosis fungoides)
b) Eczematous dermatoses
c) Lupus erythematosus
d) Cutaneous drug reactions (imatinib, captopril)
e) Insect bites

9. LICHEN MYXEDEMATOUS
• chronic, idiopathic
• characterized by lichenoid papules, nodules and/or plaques
• due to abnormal dermal mucin deposition and a variable degree of fibrosis
• fibroblast proliferation in the absence of thyroid disease.
• Two clinicopathological subsets:
– (i) a generalized papular and sclerodermoid form (scleromyxoedema of Arndt–Gottron)
– (ii) a localized papular form

10. SCLEROMYXOEDEMA
• sclerotic variant
• generalized papular eruption on a sclerodermoid background
• mucin deposition, increased fibroblast proliferation,
• fibrosis and monoclonal gammopathy .
• Systemic implications
• 30-80 years

11. • characterized by a triad of microscopic features:
– (i) a diffuse deposit of mucin
– (ii)increase in collagen deposition
– (iii)proliferation of irregularly arranged fibroblasts

12. CLINICAL FEATURES
• 2–3 mm, firm, waxy, closely spaced,
dome shaped or flat‐topped papules
• involving the dorsal aspect of the
upper limbs, head and neck region,
upper trunk and thighs.

14. • Leonine facies-Deep longitudinal erythematous folding on the forehead
• Shar-Pei sign- Deep furrowing evident on the trunk, shoulders and limbs
• Doughnut sign-Over the proximal interphalangeal joints, a central depression
surrounded by an elevated rim

15. LEONINE FACIES-

16. SHAR-PIE SIGN

17. • Doughnut sign

18. DIFFERENTIAL DIAGNOSIS
Scleroderma (systemic sclerosis)
– Absence of papules
– Antinuclear antibodies
– Anticentromere
– AntiScl70
• Scleredema
– Symmetrical non‐pitting induration of neck and upper trunk without papules
– Antecedent upper respiratory infection
– Diabetes
• Nephrogenic systemic fibrosis
– Renal dysfunction and exposure to gadolinium
– Lack of facial involvement
– monoclonal gammopathy

19. INVESTIGATIONS
• skin biopsy
• serum and electrophoresis with immunofixation
• Thyroid function tests

20. COMPLICATIONS
• Haematological- Monoclonal gammopathy,Myeloma, Hodgkin and non–Hodgkin
lymphoma,Waldenstrom macroglobulinaemia, myelomonocytic leukaemia.
• Neurological-Carpal tunnel syndrome, peripheral sensory and motor neuropathy,
central nervous system symptoms (memory loss, vertigo, gait problems, stroke,
seizures,psychosis dermatoneuro syndrome)

21. • Rheumatological- Arthralgias/arthritis, inflammatory myopathy and fibromyalgia
• Cardiovascular-Congestive heart failure, myocardial ischaemia, heart block, and
pericardial effusion
• Pulmonary Obstructive or restrictive lung involvement
• Gastrointestinal Dysphagia
• Renal Acute renal failure

22. TREATMENT LADDER
 First line
• Intravenous immunoglobulin (at a dose of 2 g/kg for 5
days for at least 6 months)
 Second line
• Thalidomide (100–400 mg/day)
• Systemic steroids
• (Both commonly in combination with intravenous
immunoglobulin rather than as monotherapy.)
 Third line
• Autologous peripheral blood stem cell transplantation
• Bortezomib and dexamethasone

23. RETICULAR
ERYTHEMATOUS
MUCINOSIS

24. DEFINITION
• Rare, chronic primary cutaneous mucinosis characterized by a persistent reticular
macular erythema or erythematous papules and plaques in the midline of the back or
chest.
• a photoaggravated disorder
• Oral contraceptives, pregnancy, menses, heat, X‐ray therapy and perspirationhave been
also implicated
• genetic predisposition

25. PATHOLOGY
• Interstitial deposits of mucin in the upper dermis
• perivascular and perifollicular T‐cell infiltratewith variable deep perivascular extension
• slight vascular dilatation
• Direct immunofluorescence is negative, but, rarely, granular deposits of
immunoglobulin M, immunoglobulin A and C3 seen at DEJ

26. CLINICAL FEATURES
• Erythematous macules and indurated papules or plaque‐like lesions
• reticular configuration
• lack of scale or other surface changes
• In the midline of the chest or back
• Atypical areas such as the arms, abdomen, face and legs are occasionally involved.
• The lesions are occasionally pruritic.

28. DIFFERENTIAL DIAGNOSIS
• lupus erythematosus tumidus.
– patients with lupus tumidus do not exhibit reticulate‐patterned lesions on the midline
– Strongly photosensitive
– have a higher rate of immune reactants on direct immunofluorescence
– have a higher tendency to recur
– present with other clinical manifestations of lupus
Seborrhoeic dermatitis
pityriasis versicolor
confluent and reticulated papillomatosis of Gougerot–Carteaud.

29. TREATMENT LADDER
• First line
• Antimalarials (hydroxychloroquine 400 mg daily)
• Second line
• Topical and systemic corticosteroid
• Topical calcineurine inhibitors
• Third line
• Phototherapy (UVA1, UVB)
• Pulsed dye laser

30. SCLEREDEMA

31. DEFINITION
• Diffuse,progressive,non‐pitting swelling and induration of the upper part of the body
caused by a thickened dermis and deposition of mucin
• Scleredema adultorum of Buschke
• Buschke disease
• Scleredema diabeticorum
• Scleredema adultorum

32. TYPES
• Diabetic-25–50%
– Slowly progressive, non‐resolving course
– Occurs in patients with poorly controlled, insulin‐dependent diabetes
• Non‐diabetic-
– 25%-Idiopathic
– 25–50% -With preceding febrile illness (poststreptococcal) and complete resolution in
months to 2 years
– 10–20%- Associated with monoclonal gammopathy
– Anecdotal- Associated with miscellaneous conditions (e.g.HIV, internal malignancies,
autoimmune)

33. PATHOPHUSIOLOGY
• Increase in type 1 collagen synthesis by dysfunctional fibroblasts
• Diabetic- accumulation of collagen due to irreversible non‐enzymatic glycosylation of
collagen and resistance to degradation by collagenase

34. CLINICAL FEATURES
• firm non‐pitting oedema
• induration typically begin on the posterior neck and spread to the upper back,
shoulders and scalp.
• Erythema and a p eau d’orange appearance of the skin
• The hands and feet are characteristically spared.
• Movement restriction comprising limited body mobility and facial expressions, or diffi
culties in mastication and articulation

36. HISTOPATHOLOGY
• Epidermis- normal
• Dermis thickened
• collagen fibres are swollen
• Acid mucopolysaccharides are found in the fenestrated spaces with special stains.
• Fat replaced by coarse collagen fibres.
• Perivascular lymphocytic infiltrate may be seen
• Fibroblast proliferation is absent.
• Mucin also accumulates in skeletal muscle and the heart.

37. TREATMENT
• First line
• Treatment of primary disease
• Phototherapy with UVA1, PUVA or NBUVB
• Physical therapy
• Second line
• Electron‐beam radiotherapy
• Photopheresis
• Intravenous immunoglobulin
• Third line
• Immunosuppresive agents

38. Thank
you